Idiopathic pulmonary fibrosis (IPF) is a severe chronic disease during which anxiety and depression are frequent comorbidities. Better knowledge of patients' expectations is needed to inform an action plan to improve medical care.
To describe feelings and expectations of patients suffering from IPF and of their carers about antifibrotic therapy and compare them to what is perceived by their pulmonologist.
National prospective study on practices and perceptions. Specific questionnaires were e-mailed to all 3276 pulmonologists in France who, in turn, invited patients and carers to participate in a survey.
147 pulmonologists, 161 patients and 144 carers participated in the survey. The role of the carer was evaluated as "important" or "very important" by more than 90% of participants, i.e. pulmonologists, patients or carers. Inconsistencies between how patients felt and how pulmonologists perceived them were identified 88% of patients responded that they understood quite well what IPF is (vs. 75% of patiew pulmonologists perceived them. Taking into account the expectations and needs of patients may allow healthcare professionals to better address their needs and priorities.
The feelings of patients suffering from IPF regarding their disease and treatment globally proved more positive compared with how pulmonologists perceived them. Taking into account the expectations and needs of patients may allow healthcare professionals to better address their needs and priorities.Thermoregulation is an important factor that could have physiological consequences on pre-clinical research outcomes. Simply housing mice at thermoneutral temperature has been shown to prevent the well-established loss of cancellous bone that is typical in growing mice. In this study, active tissue formation was induced by non-invasive tibial loading in female mice and combined with raloxifene treatment to assess whether temperature could enhance their combined effects on bone morphology and mechanical properties. It was hypothesized that by removing the cold stress under which normal lab mice are housed, a metabolic boost would allow for further architectural and mechanical improvements in mice exposed to a combination of tibial loading and raloxifene. Ten-week old female C57BL/6J mice were treated with raloxifene, underwent tibial loading to a maximum tensile stress of 2050 με, and were housed in thermoneutral conditions (32 °C) for 6 weeks. We investigated bone morphology through microcomputed tomography (μCT), mechanical properties via four-point bending, and fracture toughness testing. Results confirmed previous work showing a combined effect of external loading and raloxifene which led to greater improvements in most properties than either individual treatment. Counter to the hypothesis, temperature had modest effects on body weight, overall bone size, and trabecular architecture, and most effects were detrimental. Thermoneutrality had no impact on mechanical integrity or fracture toughness. In most cases, the magnitude of temperature-based effects were less robust than either RAL treatment or loading.Patients with osteoporosis-associated WNT1 or PLS3 mutations have unique bone histomorphometric features and osteocyte-specific hormone expression patterns.
To investigate the effects of WNT1 and PLS3 mutations on bone material properties.
Transiliac bone biopsies were evaluated by quantitative backscattered electron imaging, immunohistochemistry, and bone histomorphometry.
Ambulatory patients.
Three pediatric and eight adult patients with WNT1 or PLS3 mutations.
Bone mineralization density distribution and osteocyte protein expression was evaluated in 11 patients and repeated in six patients who underwent repeat biopsy after 24months of teriparatide treatment.
Bone mineralization density distribution and protein expression.
Children with WNT1 or PLS3 mutations had heterogeneous bone matrix mineralization, consistent with bone modeling during growth. Bone matrix mineralization was homogenous in adults and increased throughout the age spectrum. Teriparatide had very little effect on matrix mineng. The lack of change in matrix mineralization in response to teriparatide, despite clear changes in osteocyte lacunae size and protein expression, suggests that altered WNT1 and PLS3 expression may interfere with coupling of osteocyte, osteoblast, and osteoclast function. Further studies are warranted to determine the mechanism of these changes.During aging, there is a normal and mild loss in kidney function that leads to abnormalities of the kidney-bone metabolic axis. In the setting of increased phosphorus intake, hyperphosphatemia can occur despite increased concentrations of the phosphaturic hormone FGF23. This is likely from decreased expression of the FGF23 co-receptor Klotho (KL) with age; however, the roles of age and sex in the homeostatic responses to mild phosphate challenges remain unclear.
Male and female 16-week and 78-week mice were placed on either normal grain-based chow or casein (higher bioavailable phosphate) diets for 8weeks. Gene expression, serum biochemistries, micro-computed tomography, and skeletal mechanics were used to assess the impact of mild phosphate challenge on multiple organ systems. https://www.selleckchem.com/products/th-257.html Cell culture of differentiated osteoblast/osteocytes was used to test mechanisms driving key outcomes.
Aging female mice responded to phosphate challenge by significantly elevating serum intact FGF23 (iFGF23) versus control diet; phosphate challenge to maintain blood phosphorus versus young female and young/old male mice, potentially due to direct estradiol effects on osteocytes. Thus, the control of phosphate intake during aging could have modifiable outcomes for FGF23-related phenotypes.
Our study demonstrates that aging female mice upregulate FGF23 to a greater degree during a mild phosphate challenge to maintain blood phosphorus versus young female and young/old male mice, potentially due to direct estradiol effects on osteocytes. Thus, the control of phosphate intake during aging could have modifiable outcomes for FGF23-related phenotypes.